CAVIN1

Chr 17

caveolae associated protein 1

Also known as: CAVIN, CGL4, FKSG13, PTRF, cavin-1

This gene encodes a protein that enables the dissociation of paused ternary polymerase I transcription complexes from the 3' end of pre-rRNA transcripts. This protein regulates rRNA transcription by promoting the dissociation of transcription complexes and the reinitiation of polymerase I on nascent rRNA transcripts. This protein also localizes to caveolae at the plasma membrane and is thought to play a critical role in the formation of caveolae and the stabilization of caveolins. This protein translocates from caveolae to the cytoplasm after insulin stimulation. Caveolae contain truncated forms of this protein and may be the site of phosphorylation-dependent proteolysis. This protein is also thought to modify lipid metabolism and insulin-regulated gene expression. Mutations in this gene result in a disorder characterized by generalized lipodystrophy and muscular dystrophy. [provided by RefSeq, Nov 2009]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtLipodystrophy, congenital generalized, 4

Clinical highlights

Gene-disease validity (ClinGen)
lipodystrophy · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
24
Pubs (1 yr)
P/LP submissions
P/LP missense
1.08
LOEUF
GOF
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.08LOEUF
pLI 0.005
Z-score 1.40
OE 0.51 (0.271.08)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
1.38Z-score
OE missense 0.76 (0.670.85)
195 obs / 257.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.51 (0.271.08)
00.351.4
Missense OE?0.76 (0.670.85)
00.61.4
Synonymous OE?0.88
01.21.6
LoF obs/exp: 5 / 9.7Missense obs/exp: 195 / 257.1Syn Z: 1.08

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CAVIN1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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